RESULTS
Twenty-one patients were diagnosed with CLE at our center between 2013
and 2020. One patient did not attend the follow-up meetings; therefore,
20 patients were ultimately included in the study. The median age at
diagnosis was 3.2 years (range, 1 day–17 years), and the median age at
admission to our clinic was 4.53 years (range, 21 days–17 years). Nine
of the patients (45%) were female, and 11 (55%) were male. Seven
(35%) patients had no symptoms at admission to our clinic. Fourteen
patients (87.5%) presented with cough, and peroral antibiotics were
administered with the diagnosis of pneumonia; before admission to our
clinic.
Two patients (10%) were diagnosed with CXR, and thorax ultrasonography
was performed during the postnatal period, with cystic lesions observed
in the lungs in the fetal ultrasound. Three patients (15%) were
diagnosed with suspected lung infection and moaning during the neonatal
period, four patients (20%) were diagnosed with foreign body
aspiration, and nine patients (45%) were diagnosed with recurrent
and/or intractable lung infection.
One patient (5%) had a normal CXR and was diagnosed after a trauma. One
patient was diagnosed with incidental CLE by CT performed because of
cavities in the lung after pneumonia. With the exception of these two
patients, all patients presented with localized increased aeration on
the CXR. In the CXR and CT, two patients (10%) presented with
mediastinal shift and secondary atelectasis, and three patients (15%)
presented with mild mediastinal/tracheal deviation. All but three
patients (15%) underwent thoracic CT before FB. On CT, left upper lobe
10 (50%), right middle lobe 3 (15%), right upper lobe 2 (10%), left
lingula 1 (5%), left lower lobe 1 (5%) which may be compatible with
CLE. There were two patients (%10) with multiple lesions which may be
compatible with CLE. One 1 (5%) patient with right emphysematous areas
which were observed in the upper and right middle lobes , and 1(5%)
patient with bilateral emphysematous areas in the left lower and right
upper lobes. CT demonstrated vascular compression in one (5%) patient
(Table 1). None of the patients underwent ventilation-perfusion
scintigraphy.
All the patients underwent FB to rule out primary and secondary causes
that could lead to increased aeration. Macroscopically, FB revealed
malacia in eight patients (40%), mucosal folds in seven patients
(35%), and fish-mouth deformity in five patients (25%). There was
accompanying bronchial atresia in two patients and a mild vascular
pulsatile mass in one patient (Table 1). There was no microbiologically
significant growth in the bronchoalveolar lavage samples, and the
cytopathological examination results were normal.
Two patients(10%) who were followed up with a diagnosis of asthma
underwent inhaled steroid therapy, and two patients(10%) were diagnosed
with wheezing. One patient (5%) was diagnosed with tethered cord
syndrome, one patient (5%) was diagnosed with a laryngeal cleft, and
one patient (5%) was diagnosed with familial dystonia; 3 patients
(15%) presented with CHD. Echocardiography revealed patent ductus
arteriosus in one patient (5%), secundum atrial septal defect in one
patient (5%), and a small muscular ventricular septal defect in one
patient (5%). The patients diagnosed with CHD did not require medical
or surgical treatment, and there was no statistically significant
difference between the two groups in terms of comorbid diseases (Table
1).
Lobectomy was performed by the surgical department for four symptomatic
patients. The first patient was admitted to the pediatric intensive care
unit for respiratory distress and pneumonia at the age of three months
and for increased aeration in the upper zone of the left lung, causing
right upper atelectasis and a mediastinal shift as detected in the
patient’s CXRs. The patient was admitted due to their need for invasive
positive mechanical ventilation. His left upper lobe was markedly
malastic as shown by FB, was diagnosed with CLE and underwent left upper
lobectomy. While the second patient was being followed up in the
neonatal intensive care unit with 2 L/min of oxygen support through a
nasal cannula, he was referred to our clinic as an outpatient at the age
of five months with an total hyperinflation of the left lung that did
not improve and caused mediastinal shifting. The patient whose FB
revealed a fish-mouth deformity in the left lower lobe underwent a left
lower lobectomy. The third patient was examined at four months of age
due to coughing and wheezing; increased ventilation was observed in the
patient’s left lung, causing mediastinal shifting. The FB revealed that
the left upper lobe had a fish-mouth shape and that there was a mucosal
flap in the lateral part of the lesion. The fourth patient was examined
for a lung infection at the age of 15 days and was followed up on an
outpatient basis due to increased ventilation in their left lung,
causing a mediastinal shift in the patient’s CXRs. The FB revealed a
significant fish-mouth deformity in the left upper lobe. Both third and
fourth patients with mediastinal shift and frequent lung infections
underwent left upper lobectomy within two weeks after the diagnosis.
The pathology evaluation of the lobectomy specimens found enlargement of
alveolar structures in four specimens and immature bronchial cartilages
in two specimens. Concomitant pulmonary sequestration, congenital airway
malformation, and malignancy were not observed.
Although most of the patients who underwent surgery were male, there was
no statistically significant difference in sex among the patients who
were followed up conservatively. The four patients who underwent
lobectomy at diagnosis had a median age of 1.9 months (range, 1 day–4
months), and the most common symptoms were respiratory distress,
tachypnea, and retraction. The patients who were followed up
conservatively had a median age of 3.16 years (4 days–17 years) at
diagnosis, and the most common symptoms were cough. Of the four patients
who underwent surgery, three patients (75%) had left upper lobe
involvement, and one patient (25%) had left lower lobe involvement. The
mediastinal shift was observed in all patients, and secondary
atelectasis was observed in two patients in the imaging and in four
patients who underwent surgery. Respiratory distress and mediastinal
shift were more prominent in the patients who underwent surgery than the
patients who were followed up conservatively and diagnosed at an early
age (p = 0.001, p = 0.049, p = 0.001, respectively). Although the rate
of left upper lobe involvement was higher for the patients who underwent
surgery than for those treated conservatively, the difference was not
statistically significant (p = 0.067; Table 1).
Of the patients with CLE who were followed up conservatively after
diagnosis, four were able to cooperate with the spirometry in the
follow-up, one patient had normal spirometry, one had mild obstruction,
and two had mild restriction. The patients who underwent surgical
lobectomy were aged between 1 and 4 years at their last visit in 2020;
therefore, spirometry could not be performed (Table 2).
The median follow-up period was 23.2 months (8 months–7 years). In the
check-ups of the four patients who underwent lobectomy, their complaints
significantly regressed, their growth and development were normal, and
they did not experience pneumonia during their follow-up. There were
also no changes except for postoperative changes in their CXRs and
increased compensatory aeration on the operated side. Sixteen patients
who were conservatively followed up had no complaints in their check-ups
and did not have pneumonia or asthma attacks. One patient presented with
linear atelectasis, which improved with physiotherapy and postural
drainage. None of the patients required oxygen, and none were
malnourished. In the last visit, the increase in aeration in the
check-up CXRs regressed in all patients and was highly evident in two
patients(12.5%; Table 3). None of the patients who were conservatively
followed up or who underwent surgery required a repeat CT in their
follow-up. There were no deaths in the early or late postoperative
period in any of the patient groups.